How I treat prolymphocytic leukemia

Lack of durable disease control with chemotherapy for mycosis fungoides and Sézary syndrome: a comparative study of systemic therapy

“…In appropriate patients, AlloSCT may be the best therapeutic option for an effective and durable remission. Indeed, akin to this strategy used in T-cell prolymphocytic leukemia, 54 we are exploring the role of initial biological control with alemtuzumab followed by planned AlloSCT, particularly in patients with SS.…”

B-cell prolymphocytic leukemia: a specific subgroup of mantle cell lymphoma

“…The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show prolonged survival. 1,2 Several studies have shown that in more than 20% of patients originally diagnosed with B-PLL, a translocation t(11;14)(q13;q32) involving the IGH and CCND1 genes is detectable. B-PLL patients with t(11;14) appeared to be of younger age and showed male predominance, extranodal involvement, and a slightly different immunophenotype (CD5 1 and strong surface membrane [Sm] immunoglobulin…”

“…5 The diagnosis of B-PLL is mainly based on clinical and morphological data (ie, .55% prolymphocytes). 1 Correctly diagnosing the patient can, however, be difficult, as B-PLL has similarities with other mature B-cell malignancies; not only MCL but also rare cases of chronic lymphocytic leukemia (CLL), hairy cell leukemia-variant, and splenic marginal zone lymphoma. [6][7][8] Although immunophenotyping may support a diagnosis of B-PLL, a B-PLL-specific immunophenotype has not been identified yet.…”

T-cell prolymphocytic leukemia in an adolescent with ataxia-telangiectasia: novel approach with a JAK3 inhibitor (tofacitinib)

“…18 Longer survival is seen with hematopoietic cell transplantation; however, 33% to 47% of patients relapse within 36 months and treatment-related mortality is almost as high. 3 AT patients face surplus treatment-related toxicities 4 due to underlying hypersensitivity to DNA-damaging agents.…”